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November 1960

Polycythemia Vera: A Review of Fifty Cases with Emphasis on the Risk of Surgery

Author Affiliations

Charlottesville, Va.

From the Department of Internal Medicine, University of Virginia School of Medicine, and the University of Virginia Hospital, Charlottesville, Va. Present address: Massachusetts Memorial Hospitals, Boston (Dr. Rigby).

Arch Intern Med. 1960;106(5):622-627. doi:10.1001/archinte.1960.03820050034007

Introduction  Polycythemia vera was first described by Vaquez in 1892.1 Osler, in 1903, collected 6 cases and proposed that this syndrome constituted a new clinical entity.2 At the present time polycythemia vera is usually classified as one of the myeloproliferative syndromes,3,4 because apparent transformations among polycythemia vera, acute leukemia, chronic myelocytic leukemia, myelofibrosis, and Digulielmo's syndrome have been observed; however, the cause of all these syndromes remains obscure. Polycythemia vera has been the subject of some excellent reviews.5-7The purpose of this paper is to review the clinical course, complications, and prognosis of polycythemia vera as exemplified by a group of 50 patients, with particular reference to the problem of surgical risk in this disorder.

Materials and Methods  The records of 50 patients with polycythemia vera seen at the University of Virginia Hospital between the years of 1940 and 1958 have been reviewed. Information about these patients has been obtained

Vaquez, M. H.:  Concerning a Special Form of Cyanosis and Accompanying Exercise and Persistent Hyperglobulia ,  Compt. rend. Soc. de Biol. 4:384, 1892.
Osier, W.:  Chronic Cyanosis with Polycythemia and Enlarged Spleen: A New Clinical Entity ,  Am. J.M. Sc. 126:187, 1903.Crossref
Dameshek, W.:  Some Speculations on the Myeloproliferative Syndromes ,  Blood 6:372, 1951.
Dameshek, W., and Baldini, M.:  The Di-Guglielmo Syndrome ,  Blood 13:192, 1958.
Harrop, G. A., Jr.:  Polycythemia ,  Medicine 7:291, 1928.
Lawrence, J. H.; Berlin, N. I., and Huff, R. L.:  The Nature and Treatment of Polycythemia: Studies on 263 Patients ,  Medicine 32: 323, 1953.Crossref
Pike, G. M.:  Polycythemia Vera ,  New England J. Med. 258:1250, 1958.Crossref
Dameshek, W., and Henstell, H. H.:  Diagnosis of Polycythemia ,  Ann. Int. Med. 13:1360, 1940.Crossref
Masouredis, S. P., and Lawrence, J. H.:  The Problem of Leukemia in Polycythemia Vera ,  Am. J.M. Sc. 233:268, 1957.Crossref
Tinney, W. S., et al.:  Cardiac Disease and Hypertension in Polycythemia Vera ,  Proc. Staff Meet. Mayo Clin. 20:306, 1945.
Wasserman, L. R.:  Polycythemia Vera—Its Course and Treatment: Relation to Myeloid Metaplasia and Leukemia ,  Bull. New York Acad. Med. 30:343, 1954.
Schwartz, S. O., and Ehrlich, L.:  The Relationship of Polycythemia Vera to Leukemia: A Critical Review ,  Acta haemat. 4:129, 1950.Crossref
Stroebel, C. F.; Hall, B. E., and Pease, G. L.:  Evaluation of Radiophosphorus Therapy in Primary Polycythemia ,  J.A.M.A. 146:1301, 1951.Crossref
Skversky, N.; Mendell, T. H., and Frumin, A. M.:  Polycythemia Vera Terminating in Acute Leukemia ,  A.M.A. Arch. Int. Med. 96:565, 1955.Crossref
Calabresi, P., and Meyer, O. O.:  Polycythemia Vera: II. Course and Therapy ,  Ann. Int. Med. 50:1203, 1959.Crossref
Burnet, M.:  Leukemia as a Problem in Preventive Medicine ,  New England J. Med. 259:423, 1958.Crossref
Videbaek, A.:  Polycythaemia Vera: Course and Prognosis ,  Acta med. scandinav. 138:179, 1950.Crossref
Wasserman, L. R.; Lawrence, J. H.; Berlin, N. T.; Dobson, R. I., and Estres, S.:  Bone Marrow Picture in Polycythemia Vera Before and After Treatment with Radioactive Phosphorus ,  Acta med. scandinav. 143:442, 1952.Crossref
Wiseman, B. K.; Rohn, R. J.; Bouroncle, B. A., and Myers, W. G.:  Treatment of Polycythemia Vera with Radioactive Phosphorus ,  Ann. Int. Med. 34:311, 1951.Crossref
Spurr, C. L.; Smith, T. R.; Block, M. H., and Jacobson, L. O.:  A Clinical Study of the Use of Nitrogen Mustard Therapy in Polycythemia Vera.  J. Lab. & Clin. Med. 35:252, 1950.
Rosenthal, N., and Rosenthal, R. L.:  Treatment of Polycythemia Vera with Triethylene Melamine: Summary of 30 Cases ,  A.M.A. Arch. Int. Med. 90:379, 1952.Crossref
Wald, N.; Hoshino, T., and Sears, M. E.:  Therapy of Polycythemia Vera with Myleran ,  Blood 13:757, 1958.
Louis, J.:  Treatment of Polycythemia Vera with Myleran: Preliminary Report; abstracted ,  J. Lab. & Clin. Med. 50:928, 1957.
Louis, J.:  Treatment of Polycythemia Vera with Busulfan (Myleran) ,  J.A.M.A. 168:1880, 1958.
Norman, I. L., and Allen, E. V.:  The Vascular Complications of Polycythemia ,  Am. Heart J. 13:257, 1937.
Neubardt, S., and Siegman, F.:  Hemorrhagic Tendency in Polycythemia Vera ,  A.M.A. Arch. Surg. 70:257, 1955.
Burris, M. B., and Arrowsmith, W. R.:  Vascular Complications of Polycythemia Vera Study of 68 Cases ,  S. Clin. North America 33: 1023, 1953.
Whitelaw, D. M., and Thomas, G. C.:  Massive Subcutaneous Haemorrhage in Polycythemia Vera ,  Canad. M.A.J. 72:128, 1955.
Rosenthal, R. L.:  Blood Coagulation in Leukemia and Polycythemia: Value of the Heparin Clotting Time and Clot Retraction Rate ,  J. Lab. & Clin. Med. 34:321, 1949.
Henstell, H. H.; Henstell, I. S., and Ornitz, E.:  The Electrolytic Resistance of the Blood Clot in Polycythemia Vera Before and After Radiation and Its Relationship to Clot Retraction ,  Am. J. Clin. Path. 21:820, 1951.
Spaet, T. H.; Bauer, S., and Melamed, S.:  Hemorrhagic Thrombocythemia: Blood Coagula- tion Disorder ,  A.M.A. Arch. Int. Med. 98:377, 1956.
Levin, W. C., et al.:  The Mechanism of Hemorrhagic Manifestations Associated with Thrombocythemia ,  J. Lab. & Clin. Med. 46:930, 1955.
Mortensin, O.:  Thrombocythemia Hemorrhagica ,  Acta med. scandinav. 129:547, 1948.
Tinney, W. S., et al.:  Hematologic Complications of Polycythemia Vera ,  Proc. Staff Meet. Mayo Clin. 18:227, 1943.